Acute chest syndrome and sickle cell disease: What is the link? 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It involves the sudden onset of respiratory symptoms, which may lead to lung injury. SCD is an inherited disorder that affects red blood cells. In people with SCD, red blood cells are crescent- or sickle-shaped instead of disc-shaped. This impairs their ability to carry oxygen and causes them to stick together. A person with SCD may develop ACS if sickle cells stick together to form a blood clot in the small blood vessels within the lungs. Other possible causes include viral and bacterial lung infections and postsurgical complications. The article below takes an in-depth look at ACS, including its causes, treatment, and prevention. What is acute chest syndrome Share on Pinterestsukanya sitthikongsak/Getty ImagesACS is a serious and potentially life threatening condition involving sudden, severe respiratory symptoms and reduced blood oxygen levels. The condition is a potential complication of SCD. According to the Centers for Disease Control and Prevention (CDC), the most common symptoms of ACS include:chest pain when breathingcoughingshortness of breathhypoxemia, which is the medical term for a low level of oxygen in the bloodfever How does SCD cause ACS Red blood cells contain a protein called hemoglobin, which binds to oxygen. Healthy red blood cells are disc-shaped, allowing them to move freely through blood vessels to deliver oxygen to the body’s tissues and organs. In those with SCD, the hemoglobin inside red blood cells is abnormal and causes the cells to take on a characteristic sickle shape. These cells do not move through the blood vessels in the typical way and have a tendency to clump together. A person with SCD may develop ACS as a result of sickle cells blocking a pulmonary blood vessel within the lungs. The Sickle Cell Disease Association of America notes that oxygen deprivation within the lungs can result in permanent lung damage. In some cases, ACS is life threatening. Other causes and contributing factors Various factors can cause or contribute to ACS in SCD. Examples include:a lung infection, such as viral or bacterial pneumoniaasthmaoversedationpostsurgical complications In children, doctors are able to identify the cause of ACS in about 40% of cases. In the other cases, the triggering event is unclear. Risk factors for ACS According to the National Heart, Lung, and Blood Institute, more than 100,000 people in the United States have SCD. There are several types of SCD, each of which involves different gene mutations. According to a 2022 literature review, people with certain genotypes — hemoglobin SS (Hb SS) and Hb S-beta0-thalassemia — have an increased risk of developing ACS. Some additional factors that may increase a person’s risk of developing ACS include:asthmarespiratory infectionssmoking or exposure to secondhand smokecold temperatures Diagnosis A diagnosis of ACS relies on both clinical symptoms and imaging tests. Clinical symptoms that may indicate a diagnosis of ACS include:chest painincreased breathing effortchest sounds, such as coughing, wheezing, or ralesfever above 38.5°Chypoxemia Doctors may perform several tests to help rule out other illnesses and confirm a diagnosis of ACS. Examples include:Chest X-ray: This imaging test can help doctors identify pulmonary infiltrates, which are substances within the lungs, such as pus, blood, or protein. CT scan: Doctors can also use this test to look for the presence of new pulmonary infiltrates, which must be in at least one lung segment for a person to receive a diagnosis of ACS.Blood gas analysis: This test measures oxygen levels in the blood. It can help doctors detect hypoxemia. Treatment Without treatment, ACS may progress rapidly. Early treatment reduces the risk of complications and death. Most people with ACS require hospitalization for careful respiratory monitoring and treatment. According to a 2017 review, treatment may include the following:IV fluidspain medicationantibioticsincentive spirometry, which is a technique to encourage deep breathingoxygen therapyrespiratory support, such as using a ventilator for impaired breathing a blood transfusion Prevention An individual cannot eliminate all risk factors for ACS. For example, people with certain genotypes of SCD have an increased risk of developing ACS. This is a nonpreventable risk factor. However, people can take steps to reduce their risk of developing ACS. These include:taking precautions to reduce the risk of lung infections, such as staying up to date on vaccinations for pneumonia and influenzaworking with a doctor to develop an effective treatment plan for preventing asthma attacks, if asthma is present using an incentive spirometry device during hospitalization A 2017 study notes that almost half of all ACS cases develop during hospitalization. In this study, the frequency of an ACS diagnosis decreased from 22% to 12% after implementing a protocol for using incentive spirometry during hospital stays. Outlook Among people with SCD, ACS is the second most common cause of hospitalization and one of the most common causes of death. The condition has a mortality rate of 4.3% in adults and 1.1% in children. The outlook for people with ACS varies according to the nature and extent of any complications. Possible complications include:respiratory failureacute respiratory distress syndromedamage to the lung tissuesevere pain The condition can also be fatal. According to the British Society for Haematology (BSH), a person who develops ACS will require follow-up treatment, which may include blood transfusions or the chemotherapy agent hydroxycarbamide, which is also known as hydroxyurea. Summary Acute chest syndrome is a complication of sickle cell disease. People with ACS develop sudden respiratory symptoms, including chest pain and breathing difficulties, along with coughing, wheezing, or rales. A person with SCD may develop ACS as a result of sickle cells sticking together and forming a blood clot within a pulmonary blood vessel. The condition can also occur due to a viral or bacterial infection, asthma, or complications following surgery. ACS is a severe and potentially life threatening condition. However, people who receive prompt treatment tend to have a much more favorable outlook. As such, it is important that people with SCD familiarize themselves with the symptoms of ACS so that they can recognize and act on the warning signs, should they occur. Last medically reviewed on June 28, 2022COPDGeneticsRespiratoryBlood / Hematology 11 sourcescollapsedMedical News Today has strict sourcing guidelines and draws only from peer-reviewed studies, academic research institutions, and medical journals and associations. We avoid using tertiary references. We link primary sources — including studies, scientific references, and statistics — within each article and also list them in the resources section at the bottom of our articles. You can learn more about how we ensure our content is accurate and current by reading our editorial policy.Complications & treatments. (n.d.). https://www.sicklecelldisease.org/treatments/Farooq, S., et al. (2018). Acute chest syndrome in sickle cell disease [Abstract]. https://www.tandfonline.com/doi/abs/10.1080/21548331.2018.1464363Field, J. J., et al. (2022). Acute chest syndrome (ACS) in sickle cell disease (adults and children). https://www.uptodate.com/contents/acute-chest-syndrome-acs-in-sickle-cell-disease-adults-and-childrenFriend, A., et al. (2021). Acute chest syndrome. https://www.ncbi.nlm.nih.gov/books/NBK441872/Howard, J., et al. (2015). Guideline on the management of acute chest syndrome in sickle cell disease. https://onlinelibrary.wiley.com/doi/10.1111/bjh.13348Jain, S., et al. (2017). Acute chest syndrome in children with sickle cell disease. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5733742/Management of acute chest syndrome in sickle cell disease. (2015). https://b-s-h.org.uk/guidelines/guidelines/management-of-acute-chest-syndrome-in-sickle-cell-disease/Niss, O., et al. (2017). Prevention of acute chest syndrome by implementing a standardized process to improve incentive spirometry use in hospitalized patients with sickle cell disease. https://ashpublications.org/blood/article/130/Supplement%201/132/116311/Prevention-of-Acute-Chest-Syndrome-By-ImplementingOgu, U. O., et al. (2020). Management of sickle cell disease complications beyond acute chest syndrome. https://www.dovepress.com/management-of-sickle-cell-disease-complications-beyond-acute-chest-syn-peer-reviewed-fulltext-article-JBMSickle cell disease (SCD). (2022). https://www.cdc.gov/ncbddd/sicklecell/index.htmlWhat is sickle cell disease? (2022).https://www.nhlbi.nih.gov/health/sickle-cell-diseaseFEEDBACK:Medically reviewed by Angelica Balingit, MD — By MaryAnn De Pietro, CRT on June 28, 2022 Latest newsWhat sets 'SuperAgers' apart? 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